Frontotemporal Dementia

A dementia that causes changes to language and behaviour

Also known as frontotemporal lobal degeneration, or frontal lobe disorder, and previously known as Pick’s disease, these terms refer to any dementia that effects the frontal and temporal lobes of the brain, which are responsible for language skills, emotional responses, and behaviour.

In frontotemporal dementia, damage to nerve cells in one or both lobes causes the lobe to shrink (atrophy). Depending on the lobe affected, this can cause significant changes to personality or cause the person to lose language.

This type of dementia differs from most other types of dementia in that the onset of symptoms usually starts between the ages of 40 and 75, rather than over 65 years of age. Frontotemporal dementia is rarer than the most common forms of dementia; Alzheimer’s disease and vascular dementia.

Types of Frontotemporal Dementia

Depending on the area affected, people with frontotemporal dementia may have one of three types:

Behavioural variant

Semantic dementia

Progressive non-fluent aphasia

Behavioural variantMost people with frontotemporal dementia are diagnosed with this type, which is characterised by major changes in personality and behaviour in the early stages.

Early symptoms:

Reduced initiative and increased apathy

Increasingly inappropriate and uninhibited behaviours

Repetitive or compulsive behaviours

Reduced personal hygiene

Loss of empathy and sympathy

Overeating

Lack of awareness of these changes

Semantic dementiaSymptoms for this type of frontotemporal dementia often come on slowly, sometimes over years. It is most noticeable in speech.

Early symptoms:

Trouble understanding words

Inability to recognise familiar people or objects

Not using joining words such as ‘to’,’ from’, or ‘the’

Problems finding the right words while speaking

Conversation that is grammatically correct but unrelated to the conversation topic

Progression

Each person with frontotemporal dementia will have a different experience of the illness, as symptoms can vary from person to person, hour to hour.

As frontotemporal dementia progresses, the symptoms between the three types become harder to distinguish, as the disease moves to other parts of the brain. People with moderate or severe frontotemporal dementia may experience:

Profound behaviour problems including apathy, disinhibition, and loss of empathy

Severe language difficulty or loss

Memory loss

Difficulty recognising friends and family

Weight gain due to overeating

Loss of movement, requiring a wheelchair

At the end stage of frontotemporal dementia, 24 hour care is usually required because of the physical decline making eating, moving, using the toilet, showing, and dressing difficult. Life expectancy is around five to ten years, but will differ from person to person.

Causes

Genetics plays a stronger role in frontotemporal dementia than other dementias, with around 50 per cent of people having a family history of the disease. This does not mean that if a parent had frontotemporal dementia you will develop it.

A variety of mutations of specific genes have been found to cause different subtypes of frontotemporal dementia, however half of the people with this form of dementia have no family history of the disease.

Frontotemporal dementia can be caused by accumulation of one of two different proteins

Tau protein

TDP-43 protein

In some cases, the affected part of the brain contains abnormal tau protein filled structures that develop within brain cells. These are known as Pick bodies. While frontotemporal dementia used to be known as Pick’s disease, this term is now reserved for the subtype that actually features these microscopic abnormal structures.

Treatment

There are no current cures for frontotemporal dementia, however there are a range of therapies that can assist with symptoms, such as: